Further experience with the operative management of asphyxiating thoracic dystrophy after pectus repair.

BACKGROUND/PURPOSE: Asphyxiating thoracic dystrophy (ATD) can occur years after a Ravitch-type repair of pectus excavatum, resulting in debilitating alteration in pulmonary function (PFT). An operation was devised to attempt repair of this deformity.

METHODS

After institutional review board approval, the records of 10 children (ages 9-18 years) with ATD that developed 4 to 12 years postpectus operation who underwent attempted repair of ATD were reviewed. Data obtained before ATD operation and at 6, 12, and 24 months afterward included chest computed tomography, pulmonary functions (PFT), and a quality of life questionnaire. The operation consisted of sternal split with rib graft placement to permanently hold the sternum apart.

RESULTS

All children survived and the bone grafts healed solidly. Computed tomography showed a change from a flat to a round chest contour on cross section, with increased anteroposterior dimension. Two patients had no change in PFT at 24 months whereas the other 8 had 21% to 30% improvement in PFT parameters. All patients reported improved exercise tolerance, and 3 began sports activities who were previously unable to do so. Two patients on oxygen, essentially bedridden, are now active, breathing only room air. Seven of 10 patients continue to have cosmetic concerns.

CONCLUSIONS

A small population of patients who had postoperative pectus repair developed severe, debilitating ATD. The repair described improves most patients, some dramatically, but does not significantly improve cosmetic appearance. The operation is undergoing further refinement to address these issues.